Parenteral nutrition in patients with inborn errors of metabolism - a therapeutic problem.

[phenylketonuria]

Parenteral nutrition is now a standard part of supportive treatment in pediatric departments . We describe four cases in which parenteral nutrition was extremely difficult due to coincidence with inborn errors of metabolism . The first two cases was fatty acid beta -oxidation disorders associated with necrotizing enterocolitis and congenital heart disease . Thus , limitations of intravenous lipid intake made it difficult to maintain a good nutritional status . The third case was phenylketonuria associated with a facial region tumour (rhabdomyosarcoma ), in which parenteral nutrition was complicated because of a high phenylalanine content in the amino acid formulas for parenteral nutrition . The fourth patient was a child with late -diagnosed tyrosinemia type 1 , complicated with encephalopathy - during intensive care treatment the patient needed nutritional support , including parenteral nutrition - we observed amino acid formula problems similar to those in the phenylketonuria patient . Parenteral nutrition in children with inborn errors of metabolism is a rare , but very important therapeutic problem . Total parenteral nutrition formulas are not prepared for this group of diseases .